Aims

The aim of this study was to evaluate the long-term clinical and radiographic outcomes of the Birmingham Interlocking Pelvic Osteotomy (BIPO).

Aims. The purpose of this study was to explore the correlation between femoral torsion and morphology of the distal femoral condyle in patients with trochlear dysplasia and lateral patellar instability. Methods. A total of 90 patients (64 female, 26 male; mean age 22.1 years (SD 7.2)) with lateral patellar dislocation and trochlear dysplasia who were awaiting surgical treatment between January 2015 and June 2019 were retrospectively analyzed. All patients underwent CT scans of the lower limb to assess the femoral torsion and morphology of the distal femur. The femoral torsion at various levels was assessed using the a) femoral anteversion angle (FAA), b) proximal and distal anteversion angle, c) angle of the proximal femoral axis-anatomical epicondylar axis (PFA-AEA), and d) angle of the AEA–posterior condylar line (AEA-PCL). Representative measurements of distal condylar length were taken and parameters using the ratios of the bianterior condyle, biposterior condyle, bicondyle, anterolateral condyle, and anteromedial condyle were calculated and correlated with reference to the AEA, using the Pearson Correlation coefficient. Results. The femoral torsion had a strong correlation with distal condylar morphology. The FAA was significantly correlated with the ratio of the bianterior condyle (r = 0.355; p = 0.009), the AEA-PCL angle (r = 0.340; p = 0.001) and the ratio of the anterolateral condyle and lateral condyle (ALC-LC) (r = 0.309; p = 0.014). The PFA-AEA angle was also significantly correlated with the ratio of the bianterior condyle (r = 0.319; p = 0.008), the AEA-PCL angle (r = 0.231; p = 0.031), and the ratio of ALC-LC (r = 0.261; p = 0.034). In addition, the bianterior condyle ratio showed a significant correlation with the biposterior condyle ratio (r = -0.324; p = 0.027) and the AEA-PCL angle (r = 0.342; p = 0.021). Conclusion. Increased femoral torsion correlated with a prominent anterolateral condyle and a shorter posterolateral condyle compared with the medial condyle. The deformities of the anterior and posterior condyles are combined deformities rather than being isolated and individual deformities in patients with trochlear dysplasia and patella instability. Cite this article: Bone Joint J 2020;102-B(7):868–873

Aims. The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes. Methods. We retrospectively identified 20 patients with skeletal dysplasias and 292 with other syndromes (control group) who had completed surgical growth-friendly EOS treatment between 1 January 2000 and 31 December 2018. We compared radiological parameters, complications, and health-related quality of life (HRQoL) at mean follow-up of 8.6 years (SD 3.3) in the dysplasia group and 6.6 years (SD 2.6) in the control group. Results. Mean major curve correction per patient did not differ significantly between the dysplasia group (43%) and the control group (28%; p = 0.087). Mean annual spinal height increase was less in the dysplasia group (9.3 mm (SD 5.1) than in the control group (16 mm (SD 9.2); p < 0.001). Mean annual spinal growth adjusted to patient preoperative standing height during the distraction period was 11% in the dysplasia group and 14% in the control group (p = 0.070). The complication rate was 1.6 times higher (95% confidence interval (CI) 1.3 to 2.0) in the dysplasia group. The following complications were more frequent in the dysplasia group: neurological injury (rate ratio (RR) 5.1 (95% CI 2.3 to 11)), deep surgical site infection (RR 2.2 (95% CI 1.2 to 4.1)), implant-related complications (RR 2.0 (95% CI 1.5 to 2.7)), and unplanned revision (RR 1.8 (95% CI 1.3 to 2.5)). Final fusion did not provide additional spinal height compared with watchful waiting (p = 0.054). There were no significant differences in HRQoL scores between the groups. Conclusion. After growth-friendly EOS treatment, patients with skeletal dysplasias experienced a higher incidence of complications compared to those with other syndromes. Surgical growth-friendly treatment for skeletal dysplasia-associated EOS should be reserved for patients with severe, progressive deformities that are refractory to nonoperative treatment. Cite this article: Bone Joint J 2024;106-B(6):596–602

Aims. We investigated the prevalence of late developmental dysplasia of the hip (DDH), abduction bracing treatment, and surgical procedures performed following the implementation of universal ultrasound screening versus selective ultrasound screening programmes. Methods. A systematic search of PubMed, Embase, The Cochrane Library, OrthoSearch, and Web of Science from the date of inception of each database until 27 March 2022 was performed. The primary outcome of interest was the prevalence of late detection of DDH, diagnosed after three months. Secondary outcomes of interest were the prevalence of abduction bracing treatment and surgical procedures performed in childhood for dysplasia. Only studies describing the primary outcome of interest were included. Results. A total of 31 studies were identified, of which 13 described universal screening and 20 described selective screening. Two studies described both. The prevalence of late DDH was 0.10 per 1,000 live births (95% confidence interval (CI) 0.00 to 0.39) in the universal screening group and 0.45 per 1,000 live births (95% CI 0.31 to 0.61) in the selective screening group. Abduction bracing treatment was performed on 55.54 per 1,000 live births (95% CI 24.46 to 98.15) in the universal screening group versus 0.48 per 1,000 live births (95% CI 0.07 to 1.13) in the selective screening group. Both the universal and selective screening groups had a similar prevalence of surgical procedures in childhood for dysplasia being performed (0.48 (95% CI 0.32 to 0.63) vs 0.49 (95% CI 0.31 to 0.71) per 1,000 live births, respectively). Conclusion. Universal screening showed a trend towards lower prevalence of late DDH compared to selective screening. However, it was also associated with a significant increase in the prevalence of abduction bracing without a significant reduction in the prevalence of surgical procedures in childhood for dysplasia being performed. High-quality studies comparing both treatment methods are required, in addition to studies into the natural history of missed DDH. Cite this article: Bone Joint J 2023;105-B(2):198–208

Aims. Radiological residual acetabular dysplasia (RAD) has been reported in up to 30% of children who had successful brace treatment of infant developmental dysplasia of the hip (DDH). Predicting those who will resolve and those who may need corrective surgery is important to optimize follow-up protocols. In this study we have aimed to identify the prevalence and predictors of RAD at two years and five years post-bracing. Methods. This was a single-centre, prospective longitudinal cohort study of infants with DDH managed using a published, standardized Pavlik harness protocol between January 2012 and December 2016. RAD was measured at two years’ mean follow-up using acetabular index-lateral edge (AI-L) and acetabular index-sourcil (AI-S), and at five years using AI-L, AI-S, centre-edge angle (CEA), and acetabular depth ratio (ADR). Each hip was classified based on published normative values for normal, borderline (1 to 2 standard deviations (SDs)), or dysplastic (> 2 SDs) based on sex, age, and laterality. Results. Of 202 infants who completed the protocol, 181 (90%) had two and five years’ follow-up radiographs. At two years, in 304 initially pathological hips, the prevalence of RAD (dysplastic) was 10% and RAD (borderline) was 30%. At five years, RAD (dysplastic) decreased to 1% to 3% and RAD (borderline) decreased to < 1% to 2%. On logistic regression, no variables were predictive of RAD at two years. Only AI-L at two years was predictive of RAD at five years (p < 0.001). If both hips were normal at two years’ follow-up (n = 96), all remained normal at five years. In those with bilateral borderline hips at two years (n = 21), only two were borderline at five years, none were dysplastic. In those with either borderline-dysplastic or bilateral dysplasia at two years (n = 26), three (12%) were dysplastic at five years. Conclusion. The majority of patients with RAD at two years post-brace treatment, spontaneously resolved by five years. Therefore, children with normal radiographs at two years post-brace treatment can be discharged. Targeted follow-up for those with abnormal AI-L at two years will identify the few who may benefit from surgical correction at five years’ follow-up. Cite this article: Bone Joint J 2024;106-B(7):744–750

Aims. The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. Patients and Methods. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

We made a prospective longitudinal clinical and radiological study of 18 children diagnosed as having dysplasia epiphysealis capitis femoris. Half the cases were bilateral. Boys were affected five times more often than girls. There were no symptoms or clinical signs in most but some of the bilateral cases had an inconsistent waddling gait. The imaging studies suggest that the cartilaginous proximal femoral epiphysis is hypoplastic, with delayed appearance of single or multiple ossification centres. Progressive improvement occurred and at an average age of five years and six months, there was complete fusion of all the ossific nuclei and normal density and texture of the epiphyseal bone. The end result was a round epiphysis with a slightly diminished height. The dysplasia is attributed to focal hypoplasia of the proximal femoral epiphysis

Aims. The Oswestry-Bristol Classification (OBC) is an MRI-specific assessment tool to grade trochlear dysplasia. The aim of this study is to validate clinically the OBC by demonstrating its use in selecting treatments that are safe and effective. Methods. The OBC and the patellotrochlear index were used as part of the Oswestry Patellotrochlear Algorithm (OPTA) to guide the surgical treatment of patients with patellar instability. Patients were assigned to one of four treatment groups: medial patellofemoral ligament reconstruction (MPFLr); MPFLr + tibial tubercle distalization (TTD); trochleoplasty; or trochleoplasty + TTD. A prospective analysis of a longitudinal patellofemoral database was performed. Between 2012 and 2018, 202 patients (233 knees) with a mean age of 24.2 years (SD 8.1), with recurrent patellar instability were treated by two fellowship-trained consultant sports/knee surgeons at The Robert Jones and Agnes Hunt Orthopaedic Hospital. Clinical efficacy of each treatment group was assessed by Kujala, International Knee Documentation Committee (IKDC), and EuroQol five-dimension questionnaire (EQ-5D) scores at baseline, and up to 60 months postoperatively. Their safety was assessed by complication rate and requirement for further surgery. The pattern of clinical outcome over time was analyzed using mixed regression modelling. Results. In all, 135 knees (mean age 24.9 years (SD 9.4)) were treated using a MPFLr. Ten knees (7.4%) required additional surgery. A total of 50 knees (mean age 24.4 years (SD 6.3)) were treated using MPFLr + TTD. Ten (20%) required additional surgery. A total of 20 knees (mean age 19.5 years (SD 3.0)) were treated using trochleoplasty + TTD. Three patients (15%) required additional surgery. In each treatment group, there was a significant improvement in Kujala, IKDC, and EQ-5D at one year postoperatively (p < 0.001) with a recognized level of overall complication rate. Conclusion. The OBC is a valid assessment tool to grade patients with trochlear dysplasia and, when used as part of the OPTA, helps to determine treatments that are safe and effective. This fulfils the requirements for its application in mainstream clinical practice. Cite this article: Bone Joint J 2021;103-B(10):1586–1594

Metal-on-metal hip resurfacing was performed for developmental dysplasia in 96 hips in 85 patients, 78 in women and 18 in men, with a mean age at the time of surgery of 43 years (14 to 65). These cases were matched for age, gender, operating surgeon and date of operation with a group of patients with primary osteoarthritis who had been treated by resurfacing, to provide a control group of 96 hips (93 patients). A clinical and radiological follow-up study was performed. The dysplasia group were followed for a mean of 4.4 years (2.0 to 8.5) and the osteoarthritis group for a mean of 4.5 years (2.2 to 9.4). Of the dysplasia cases, 17 (18%) were classified as Crowe grade III or IV. There were five (5.2%) revisions in the dysplasia group and none in the osteoarthritic patients. Four of the failures were due to acetabular loosening and the other sustained a fracture of the neck of femur. There was a significant difference in survival between the two groups (p = 0.02). The five-year survival was 96.7% (95% confidence interval 90.0 to 100) for the dysplasia group and 100% (95% confidence interval 100 to 100) for the osteoarthritic group. There was no significant difference in the median Oxford hip score between the two groups at any time during the study. The medium-term results of metal-on-metal hip resurfacing in all grades of developmental dysplasia are encouraging, although they are significantly worse than in a group of matched patients with osteoarthritis treated in the same manner

In a longitudinal case-control study, we followed 81 subjects with dysplasia of the hip and 136 control subjects without dysplasia for ten years assessing radiological evidence of degeneration of the hip at admission and follow-up. There were no cases of subluxation in the group with dysplasia. Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission. We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip. The association of subluxation and/or associated acetabular labral tears with dysplasia of the hip may be a conditional factor for the development of premature osteoarthritis in mildly to moderately dysplastic hips

Patients with acetabular dysplasia commonly undergo peri-acetabular osteotomy after skeletal maturity to reduce the risk of the late development of osteoarthritis. Several studies have suggested that deformity of the femoral head influences the long-term outcome. We radiologically examined 224 hips in 112 patients with acetabular dysplasia and early-stage osteoarthritis. There were 103 women and nine men with a mean age of 37.6 years (18 to 49). A total of 201 hips were placed in the acetabular dysplasia group and 23 in a normal group. The centre–edge angle and acetabular head index were significantly smaller (both p < 0.001), and the acetabular angle, acetabular roof angle and roundness index were significantly greater in the acetabular dysplasia group than those in the normal group (all p < 0.001). There were significant correlations between the roundness index and other parameters. Femoral head shape may be influenced by the severity of the acetabular dysplasia. Cite this article: Bone Joint J 2013;95-B:1192–6

Aims. Some patients presenting with hip pain and instability and underlying acetabular dysplasia (AD) do not experience resolution of symptoms after surgical management. Hip-spine syndrome is a possible underlying cause. We hypothesized that there is a higher frequency of radiological spine anomalies in patients with AD. We also assessed the relationship between radiological severity of AD and frequency of spine anomalies. Methods. In a retrospective analysis of registry data, 122 hips in 122 patients who presented with hip pain and and a final diagnosis of AD were studied. Two observers analyzed hip and spine variables using standard radiographs to assess AD. The frequency of lumbosacral transitional vertebra (LSTV), along with associated Castellvi grade, pars interarticularis defect, and spinal morphological measurements were recorded and correlated with radiological severity of AD. Results. Out of 122 patients, 110 (90.2%) were female and 12 (9.8%) were male. We analyzed the radiographs of 122 hips (59 (48.4%) symptomatic left hips, and 63 (51.6%) symptomatic right hips). Average age at time of presentation was 34.2 years (SD 11.2). Frequency of LSTV was high (39% to 43%), compared to historic records from the general population, with Castellvi type 3b being the most common (60% to 63%). Patients with AD have increased L4 and L5 interpedicular distance compared to published values. Frequency of pars interarticularis defect was 4%. Intraclass correlation coefficient for hip and spine variables assessed ranged from good (0.60 to 0.75) to excellent (0.75 to 1.00). Severity of AD did not demonstrate significant correlation with frequency of radiological spine anomalies. Conclusion. Patients with AD have increased frequency of spinal anomalies seen on standard hip radiographs. However, there exists no correlation between radiological severity of AD and frequency of spine anomalies. In managing AD patients, clinicians should also assess spinal anomalies that are easily found on standard hip radiographs. Cite this article: Bone Joint J 2021;103-B(8):1351–1357

Monostotic fibrous dysplasia of the proximal femur has a variable clinical course, despite its reported limited tendency to progress. We investigated the natural history and predisposing factors for progression of dysplasia in a group of 76 patients with a mean follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented with an asymptomatic incidental lesion while 45 (59%) presented with pain or a pathological fracture. A group of 23 patients (30%) underwent early operative treatment for pain (19: 25%) or pathological fracture (4: 5%). Of the 53 patients who were initially treated non-operatively, 45 (85%) remained asymptomatic but eight (15%) needed surgery because of pain or fracture. The progression-free survival of the observation group was 81% (. sd. 6.4%) at five-years follow-up. An initial presentation of pain (p < 0.001), a limp (p < 0.001), radiological evidence of microfracture (p = 0.001) and younger age (< 17 years) (p = 0.016) were significant predisposing factors for disease progression. The risk of experiencing pain or pathological fracture is considerable in monostotic fibrous dysplasia of the proximal femur. Patients presenting with pain, a limp or radiological evidence of microfracture have a high chance of needing surgical treatment. Cite this article: Bone Joint J 2014;96-B:673–6

Aims. The objective of this study was to present the outcomes of rotational acetabular osteotomy (RAO) over a 30-year period for osteoarthritis (OA) secondary to dysplasia of the hip in pre- or early-stage OA. Methods. Between September 1987 and December 1994, we provided treatment to 47 patients (55 hips) with RAO for the management of pre- or early-stage OA due to developmental hip dysplasia. Of those, eight patients (11 hips) with pre-OA (follow-up rate 79%) and 27 patients (32 hips) with early-stage OA (follow-up rate 78%), totalling 35 patients (43 hips) (follow-up rate 78%), were available at a minimum of 28 years after surgery. Results. In the pre-OA group, the mean Merle d'Aubigné score improved significantly from 14.5 points (SD 0.7) preoperatively to 17.4 points at final follow-up (SD 1.2; p = 0.004) and in the early-stage group, the mean score did not improve significantly from 14.0 (SD 0.3) to 14.6 (SD 2.4; p = 0.280). Radiologically, the centre-edge angle, acetabular roof angle, and head lateralization index were significantly improved postoperatively in both groups. Radiological progression of OA was observed in two patients (two hips) in the pre-OA group and 17 patients (18 hips) in the early-stage group. Kaplan-Meier survival analysis, with radiological progression of OA as the primary outcome, projected a 30-year survival rate of 81.8% (95% confidence interval (CI) 0.59 to 1.00) for the pre-OA group and 42.2% (95% CI 0.244 to 0.600) for the early-stage group. In all cases, the overall survival rate stood at 51.5% (95% CI 0.365 to 0.674) over a 30-year period, and when the endpoint was conversion to total hip arthroplasty, the survival rate was 74.0% (95% CI 0.608 to 0.873). Conclusion. For younger patients with pre-OA, joint preservation of over 30 years can be expected after RAO. Cite this article: Bone Joint J 2024;106-B(5 Supple B):25–31

Aims. The aim of this study was to evaluate cartilaginous patellotrochlear congruence and patellofemoral alignment parameters after deepening trochleoplasty in severe trochlear dysplasia. Patients and Methods. The study group comprised 20 patients (two male, 18 female; mean age 24 years (16 to 39)) who underwent deepening trochleoplasty and medial patellofemoral ligament (MPFL) reconstruction for the treatment of recurrent lateral patellar dislocation due to severe trochlear dysplasia (Dejour type B to D). Pre- and postoperative MRI investigations of the study group were compared with MRI data of 20 age- and gender-matched control patients (two male, 18 female; mean age 27 years (18 to 44)) regarding the patellotrochlear contact ratio, patellotrochlear contact area, posterior patellar edge-trochlear groove ratio, and patellar tilt. Results. Preoperatively, all parameters in the study group indicated significant patellar malalignment, including a reduced patellotrochlear contact ratio (mean 0.38 (0.13 to 0.59) vs mean 0.67 (0.48 to 0.88); p < 0.001), reduced contact area (mean 128 mm. 2. (87 to 190) vs mean 183 mm. 2. (155 to 227); p < 0.001), increased patellar lateralization (mean 0.82 (0.55 to 1.0) vs mean 0.99 (0.93 to 1.1); p < 0.001) and increased patellar tilt (mean 25.8° (8.1° to 43.0°) vs mean 9.1° (0.5° to 16.8°); p < 0.001). Postoperatively, all parameters in the study group improved significantly and reached normal values compared with the control group. The patellotrochlear contact ratio increased to a mean of 0.63 (0.38 to 0.85) (p < 0.001), the contact area increased to a mean of 187 mm. 2. (101 to 255) (p < 0.001), the posterior patellar edge-trochlear groove ratio improved to a mean of 0.96 (0.83 to 0.91) (p < 0.001), and the patellar tilt decreased to a mean of 13.9° (8.1° to 22.1°) (p < 0.001) postoperatively. Conclusion. Deepening trochleoplasty and MPFL reconstruction normalized the patellotrochlear congruence and patellofemoral alignment parameters. Modifying the dysplastic trochlea involves approximating the trochlear morphology to the shape of the patella rather than inducing a patella and trochlea mismatch. Cite this article: Bone Joint J 2019;101-B:325–330

The aim of this study was to investigate the incidence of dysplasia in the ‘normal’ contralateral hip in patients with unilateral developmental dislocation of the hip (DDH) and to evaluate the long-term prognosis of such hips. A total of 48 patients (40 girls and eight boys) were treated for late-detected unilateral DDH between 1958 and 1962. After preliminary skin traction, closed reduction was achieved at a mean age of 17.8 months (4 to 65) in all except one patient who needed open reduction. In 25 patients early derotation femoral osteotomy of the contralateral hip had been undertaken within three years of reduction, and later surgery in ten patients. Radiographs taken during childhood and adulthood were reviewed. The mean age of the patients was 50.9 years (43 to 55) at the time of the latest radiological review. In all, eight patients (17%) developed dysplasia of the contralateral hip, defined as a centre-edge (CE) angle < 20° during childhood or at skeletal maturity. Six of these patients underwent surgery to improve cover of the femoral head; the dysplasia improved in two after varus femoral osteotomy and in two after an acetabular shelf operation. During long-term follow-up the dysplasia deteriorated to subluxation in two patients (CE angles 4° and 5°, respectively) who both developed osteoarthritis (OA), and one of these underwent total hip replacement at the age of 49 years. In conclusion, the long-term prognosis for the contralateral hip was relatively good, as OA occurred in only two hips (4%) at a mean follow-up of 50 years. Regular review of the ‘normal’ side is indicated, and corrective surgery should be undertaken in those who develop subluxation. Cite this article: Bone Joint J 2014; 96-B:1161–6

1. The examination of fifty-one infants presenting signs of unilateral dysplasia (preluxation) of the hip disclosed the existence of an abduction-contracture in the opposite "normal" hip. 2. The abduction-contracture was not infrequently found in the newborn, but seemed to attract attention mostly between the second and fifth month of life. It was seldom found after the sixth month. 3. The dysplasia in the contralateral hip showed the usual features. 4. The relationship between the degree of abduction-contracture and the degree of dysplasia was a direct one only occasionally; in most cases they seemed unrelated to each other. 5. Observation of these patients showed that the abduction-contracture followed a constant course towards gradual and spontaneous regression. The dysplasia progressed in one of the following directions: spontaneous recovery, rapid or slow recovery with treatment by abduction splint, subluxation, or true dislocation. 6. It is submitted that the coexistence of unilateral dysplasia of the hip and abduction-contracture in the opposite hip is not fortuitous. It is believed that the abduction-contracture determines the development of the dysplasia in the opposite hip through the faulty mechanics caused by "fixed pelvic obliquity.". 7. The discovery of unilateral abduction-contracture soon after birth should be a warning that dysplasia may develop in the opposite hip. A careful watch should therefore be kept for signs of abduction-contracture and for the later development of dysplasia

Aims. The aim of this study was to investigate the changes in femoral trochlear morphology following surgical correction of recurrent patellar dislocation associated with trochlear dysplasia in children. Patients and Methods. A total of 23 patients with a mean age of 9.6 years (7 to 11) were included All had bilateral recurrent patellar dislocation associated with femoral trochlear dysplasia. The knee with traumatic dislocation at the time of presentation or that had dislocated most frequently was treated with medial patellar retinacular plasty (Group S). The contralateral knee served as a control and was treated conservatively (Group C). All patients were treated between October 2008 and August 2013. The mean follow-up was 48.7 months (43 to 56). Axial CT scans were undertaken in all patients to assess the trochlear morphological characteristics on a particular axial image which was established at the point with the greatest epicondylar width based on measurements preoperatively and at the final follow-up. Results. Preoperatively, there were no statistically significant differences between the trochlear morphology in the two groups (sulcus angle, p 0.852; trochlear groove depth, p 0.885; lateral trochlear inclination, p 0.676; lateral-to-medial facet ratio, p 0.468; lateral condylar height, p 0.899; medial condylar height, p 0.816). Many radiological parameters of trochlear morphology were significantly different between the two groups at the final follow-up, including well-known parameters, such as the mean sulcus angle (Group S, 146.27° (. sd. 7.18); Group C, 160.61° (. sd. 9.29); p < 0.001), the mean trochlear groove depth (Group S, 6.25 mm (. sd. 0.41); Group C, 3.48 mm (. sd. 0.65); p < 0.001) and the mean lateral trochlear inclination (Group S, 20.99° (. sd. 3.87); Group C, 12.18° (. sd. 1.85); p < 0.001). Lesser known parameters such as the ratio of the lateral to medial trochlear length (Group S, 1.46 (. sd. 0.19); Group C, 2.14 (. sd. 0.42); p < 0.001), which is a measurement of facet asymmetry, and the lateral and medial condylar height were also significantly different between the two groups (p < 0.001). Conclusion. The femoral trochlear morphology can be improved by early (before epiphyseal closure) surgical correction in children with recurrent patellar dislocation associated with femoral trochlear dysplasia. Cite this article: Bone Joint J 2018;100-B:811–21

In Japan, osteoarthritis (OA) of the hip secondary to acetabular dysplasia is very common, and there are few data concerning the pathogeneses and incidence of femoroacetabular impingement (FAI). We have attempted to clarify the radiological prevalence of painful FAI in a cohort of Japanese patients and to investigate the radiological findings. We identified 176 symptomatic patients (202 hips) with Tönnis grade 0 or 1 osteoarthritis, whom we prospectively studied between August 2011 and July 2012. There were 61 men (65 hips) and 115 women (137 hips) with a mean age of 51.8 years (11 to 83). Radiological analyses included the α-angle, centre–edge angle, cross-over sign, pistol grip deformity and femoral head neck ratio. Of the 202 hips, 79 (39.1%) had acetabular dysplasia, while 80 hips (39.6%) had no known aetiology. We found evidence of FAI in 60 hips (29.7%). Radiological FAI findings associated with cam deformity were the most common. There was a significant relationship between the pistol grip deformity and both the α-angle (p < 0.001) and femoral head–neck ratio (p = 0.024). Radiological evidence of symptomatic FAI was not uncommon in these Japanese patients. Cite this article: Bone Joint J 2013;96-B:172–6

We investigated the clinical and radiological outcome of trochleaplasty for recurrent patellar dislocation in association with trochlear dysplasia in 38 consecutive patients (45 knees) with a mean follow-up of 8.3 years (4 to 14). None had recurrence of dislocation after trochleaplasty. Post-operatively, patellofemoral pain, present pre-operatively in only 35 knees, became worse in 15 (33.4%), remained unchanged in four (8.8%) and improved in 22 (49%). Four knees which had no pain pre-operatively (8.8%) continued to have no pain. A total of 33 knees were available for radiological assessment. Post-operatively, all but two knees (93.9%) had correction of trochlear dysplasia radiologically but degenerative changes of the patellofemoral joint developed in 30% (10) of the knees. We conclude that recurrent patellar dislocation associated with trochlear dysplasia can be treated successfully by trochleaplasty, but the impact on patellofemoral pain and the development of patellofemoral osteoarthritis is less predictable. Overall, subjective patient satisfaction with restored patellofemoral stability after trochleaplasty appeared to outweigh its possible sequelae

Patients with skeletal dysplasia are prone to developing advanced osteoarthritis of the knee requiring total knee replacement (TKR) at a younger age than the general population. TKR in this unique group of patients is a technically demanding procedure owing to the deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We retrospectively reviewed the outcome of 11 TKRs performed in eight patients with skeletal dysplasia at our institution using the Stanmore Modular Individualised Lower Extremity System (SMILES) custom-made rotating-hinge TKR. There were three men and five women with mean age of 57 years (41 to 79). Patients were followed clinically and radiologically for a mean of seven years (3 to 11.5). The mean Knee Society clinical and function scores improved from 24 (14 to 36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to 80) and 50 (22 to 74), respectively, at final follow-up. Four complications were recorded, including a patellar fracture following a fall, a tibial peri-prosthetic fracture, persistent anterior knee pain, and aseptic loosening of a femoral component requiring revision. Our results demonstrate that custom primary rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, with a satisfactory range of movement and improved function. It compensates for bony deformity and ligament deficiency and reduces the likelihood of corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than with primary TKR in the general population

We followed up 76 consecutive hips with symptomatic acetabular dysplasia treated by acetabular shelf augmentation for a mean period of 11 years. Survival analysis using conversion to hip replacement as an end-point was 86% at five years and 46% at ten years. Forty-four hips with slight or no narrowing of the joint space pre-operatively had a survival of 97% at five and 75% at ten years. This was significantly higher (p = 0.0007) than that of the 32 hips with moderate or severe narrowing of the joint-space, which was 76% at five and 22% at ten years. There was no significant relationship between survival and age (p = 0.37) or the pre- and post-operative centre-edge (p = 0.39) and acetabular angles (p = 0.85). Shelf acetabuloplasty is a reliable, safe procedure offering medium-term symptomatic relief for adults with acetabular dysplasia. The best results were achieved in patients with mild and moderate dysplasia of the hip with little arthritis

Since this paper was submitted for publication three additional reports have appeared, two of which represent typical examples of the syndrome. Husson and Parkman (1961) reported the case of a female infant dying at the age of four months with chondroectodermal dysplasia, obliteration of the upper labiogingival sulcus, and congenital heart disease. The heart lesions included anomalous pulmonary venous return, single atrium and a persistent left superior vena cava. There was no known consanguinity. In reviewing the cardiac lesions described in chondroectodermal dysplasia they include another case with anomalous pulmonary venous return described by Darling, Rothney and Craig (1957), which also showed chondroectodermal dysplasia and polydactyly, though details of the extracardiac malformations are not given. Nabrady (1961) described a four-year-old Hungarian girl with ectodermal defects involving the teeth and nails but not the hair, polydactyly, distal shortening of the extremities producing dwarfing, and typical radiological appearances of the long bones. There was presumptive evidence of a septal defect of the heart. There was no consanguinity, but the mother was considered to show a "forme fruste" of the condition. A necropsy report by Meitner (1961) of a newly born premature infant with multiple congenital malformations of organs of ectodermal, mesodermal and endodermal origin is of interest because these malformations included extreme polydactyly of hands and feet, absence of nails, chondrodysplasia, and trilocular heart. In many respects, however, the case is atypical of chondroectodermal dysplasia

1 . Twenty patients with fibrous dysplasia, confirmed histologically, are reported and discussed in regard to classification, etiology, pathogenesis and treatment. The various fibrous or fibrocystic lesions of bone are characterised briefly for purpose of contrast, and the position of fibrous dysplasia in this heterogeneous collection is suggested. 2. The classification of fibrous dysplasia based on the degree of skeletal involvement is used, and the diagnostic, therapeutic and prognostic implications of this classification emphasised. The authors endorse the opinion that fibrous dysplasia is a developmental defect. Clinical, histological and radiographic evidence is presented to point to the distinct evolution of the monostotic lesions, to which a positive and aggressive approach is recommended

Our aim was to determine whether abnormalities noted on MRI immediately after reduction for developmental dysplasia of the hip could predict the persistance of dysplasia and aid surgical planning. Scans of 13 hips in which acetabular dysplasia had resolved by the age of four years were compared with those of five which had required pelvic osteotomy for persisting dysplasia. The scans were analysed by two consultant musculoskeletal radiologists who were blinded to the outcome in each child. The postreduction scans highlighted a number of anatomical abnormalities secondary to developmental dysplasia of the hip, but statistical analysis showed that none were predictive of persisting acetabular dysplasia in the older child, suggesting that the factors which determine the long-term outcome were not visible on these images

There is controversy whether congenital foot abnormalities are true risk factors for pathological dysplasia of the hip. Previous United Kingdom screening guidelines considered congenital talipes equinovarus (CTEV) to be a risk factor for hip dysplasia, but present guidelines do not. We assessed the potential relationship between pathological dysplasia of the hip and fixed idiopathic CTEV. We present a single-centre 21-year prospective longitudinal observational study. All fixed idiopathic CTEV cases were classified (Harrold and Walker Types 1 to 3) and the hips clinically and sonographically assessed. Sonographic Graf Type III, IV and radiological irreducible hip dislocation were considered to be pathological hip dysplasia. Over 21 years there were 139 children with 199 cases of fixed idiopathic CTEV feet. Sonographically, there were 259 normal hips, 18 Graf Type II hips, 1 Graf Type III hip and 0 Graf Type IV hip. There were no cases of radiological or sonographic irreducible hip dislocation. Fixed idiopathic CTEV should not be considered as a significant risk factor for pathological hip dysplasia. This conclusion is in keeping with the current newborn and infant physical examination guidelines in which the only risk factors routinely screened are family history and breech presentation. Our findings suggest CTEV should not be considered a significant risk factor in pathological dysplasia of the hip. Cite this article: Bone Joint J 2014;96-B:1553–5

Aims. Acetabular dysplasia is frequently associated with intra-articular pathology such as labral tears, but whether labral tears should be treated at the time of periacetabular osteotomy (PAO) remains controversial. The purpose of this study was to compare the clinical outcomes and radiographic corrections of PAO for acetabular dysplasia between patients with and without labral tears pre-operatively. Patients and Methods. We retrospectively reviewed 70 hips in 67 patients with acetabular dysplasia who underwent PAO. Of 47 hips (45 patients) with labral tears pre-operatively, 27 (25 patients) underwent PAO alone, and were classified as the labral tear alone (LT) group, and 20 (20 patients) underwent combined PAO and osteochondroplasty, and were classified as the labral tear osteochondroplasty (LTO) group. The non-labral tear (NLT) group included 23 hips in 22 patients. Results. There were no significant differences between groups for post-operative Harris hip scores, degree of progression of osteoarthritis or rate of reoperation. The pre-operative alpha angle was significantly larger in the LTO group compared with the other groups (p < 0.0001). Conclusion. PAO provides equivalent short-term relief of pain and functional outcome in patients with or without labral tears. The rate of progression of osteoarthritis and reoperation was not significantly increased in patients with labral tears. Take home message: PAO provides equivalent short-term pain relief and functional outcomes in patients with acetabular dysplasia with and without labral tears. We did not find significantly increased risks of progression of osteoarthritis or re-operation in those with labral tears. Cite this article: Bone Joint J 2016;98-B:741–6

Untreated acetabular dysplasia following treatment for developmental dysplasia of the hip (DDH) leads to early degenerative joint disease. Clinicians must accurately and reliably recognise dysplasia in order to intervene appropriately with secondary acetabular or femoral procedures. This study sought early predictors of residual dysplasia in order to establish empirically-based indications for treatment. DDH treated by closed or open reduction alone was reviewed. Residual hip dysplasia was defined according to the Severin classification at skeletal maturity. Future hip replacement in a subset of these patients was compared with the Severin classification. Serial measurements of acetabular development and subluxation of the femoral head were collected, as were the age at reduction, type of reduction, and Tonnis grade prior to reduction. These variables were used to predict the Severin classification. The mean age at reduction in 72 hips was 16 months (1 to 46). On the final radiograph, 47 hips (65%) were classified as Severin I/II, and 25 as Severin III/IV (35%). At 40 years after reduction, five of 43 hips (21%) had had a total hip replacement (THR). The Severin grade was predictive for THR. Early measurements of the acetabular index (AI) were predictive for Severin grade. For example, an AI of 35° or more at two years after reduction was associated with an 80% probability of becoming a Severin grade III/IV hip. This study links early acetabular remodelling, residual dysplasia at skeletal maturity and the long-term risk of THR. It presents evidence describing the diagnostic value of early predictors of residual dysplasia, and therefore, of the long-term risk of degenerative change

1. The occurrence of bilateral double-layer patellae in association with multiple epiphysial dysplasia is described in three siblings. 2. Twelve cases of bilateral double-layer patellae have been reported previously, and in all there was an accompanying skeletal dystrophy which, although not diagnosed as such, is likely to have been multiple epiphysial dysplasia. 3. It is suggested that although they occur only in a minority of cases, double-layer patellae when present are a feature of considerable diagnostic value in multiple epiphysial dysplasia. Lateral radiography of the knees may therefore be of assistance in the diagnosis of multiple epiphysial dysplasia

Aims. This study aimed to explore whether intraoperative nerve monitoring can identify risk factors and reduce the incidence of nerve injury in patients with high-riding developmental dysplasia. Patients and Methods. We conducted a historical controlled study of patients with unilateral Crowe IV developmental dysplasia of the hip (DDH). Between October 2016 and October 2017, intraoperative nerve monitoring of the femoral and sciatic nerves was applied in total hip arthroplasty (THA). A neuromonitoring technician was employed to monitor nerve function and inform the surgeon of ongoing changes in a timely manner. Patients who did not have intraoperative nerve monitoring between September 2015 and October 2016 were selected as the control group. All the surgeries were performed by one surgeon. Demographics and clinical data were analyzed. A total of 35 patients in the monitoring group (ten male, 25 female; mean age 37.1 years (20 to 46)) and 56 patients in the control group (13 male, 43 female; mean age 37.9 years (23 to 52)) were enrolled. The mean follow-up of all patients was 13.1 months (10 to 15). Results. The two groups had no significant differences in preoperative data. In the monitoring group, ten nerve alerts occurred intraoperatively, and no neural complications were detected postoperatively. In the control group, six patients had neural complications. The rate of nerve injury was lower in the monitoring group than in the control group, but this did not achieve statistical significance. The degree of leg lengthening was significantly greater in the monitoring group than in the control group. In further analyses, patients who had previous hip surgery were more likely to have intraoperative nerve alerts and postoperative nerve injury. Conclusion. Nerve injury usually occurred during the processes of exposure and reduction. The use of intraoperative nerve monitoring showed a trend towards reduced nerve injury in THA for Crowe IV DDH patients. Hence, we recommend its routine use in patients undergoing leg lengthening, especially in those with previous hip surgery. Cite this article: Bone Joint J 2019;101-B:1438–1446

The aim of this study was to review the early outcome of the Femoro-Patella Vialla (FPV) joint replacement. A total of 48 consecutive FPVs were implanted between December 2007 and June 2011. Case-note analysis was performed to evaluate the indications, operative histology, operative findings, post-operative complications and reasons for revision. The mean age of the patients was 63.3 years (48.2 to 81.0) and the mean follow-up was 25.0 months (6.1 to 48.9). Revision was performed in seven (14.6%) at a mean of 21.7 months, and there was one re-revision. Persistent pain was observed in three further patients who remain unrevised. The reasons for revision were pain due to progressive tibiofemoral disease in five, inflammatory arthritis in one, and patellar fracture following trauma in one. No failures were related to the implant or the technique. Trochlear dysplasia was associated with a significantly lower rate of revision (5.9% vs 35.7%, p = 0.017) and a lower incidence of revision or persistent pain (11.8% vs 42.9%, p = 0.045). . Focal patellofemoral osteoarthritis secondary to trochlear dysplasia should be considered the best indication for patellofemoral replacement. Standardised radiological imaging, with MRI to exclude overt tibiofemoral disease should be part of the pre-operative assessment, especially for the non-dysplastic knee. Cite this article: Bone Joint J 2013;95-B:793–7

To confirm whether developmental dysplasia of the hip has a risk of hip impingement, we analysed maximum ranges of movement to the point of bony impingement, and impingement location using three-dimensional (3D) surface models of the pelvis and femur in combination with 3D morphology of the hip joint using computer-assisted methods. Results of computed tomography were examined for 52 hip joints with DDH and 73 normal healthy hip joints. DDH shows larger maximum extension (p = 0.001) and internal rotation at 90° flexion (p < 0.001). Similar maximum flexion (p = 0.835) and external rotation (p = 0.713) were observed between groups, while high rates of extra-articular impingement were noticed in these directions in DDH (p < 0.001). Smaller cranial acetabular anteversion (p = 0.048), centre-edge angles (p < 0.001), a circumferentially shallower acetabulum, larger femoral neck anteversion (p < 0.001), and larger alpha angle were identified in DDH. Risk of anterior impingement in retroverted DDH hips is similar to that in retroverted normal hips in excessive adduction but minimal in less adduction. These findings might be borne in mind when considering the possibility of extra-articular posterior impingement in DDH being a source of pain, particularly for patients with a highly anteverted femoral neck. Cite this article: Bone Joint J 2014;96-B:580–9

1. Familial metaphysial dysplasia occurring in two families is recorded. 2. One case with unusual skull changes and webbing of the digits is described in detail. 3. The similarity between the shape of the bones in familial metaphysial dysplasia and other bone disease is discussed

The dysplasia cup, which was devised as an adjunct to the Birmingham Hip Resurfacing system, has a hydroxyapatite-coated porous surface and two supplementary neutralisation screws to provide stable primary fixation, permit early weight-bearing, and allow incorporation of morcellised autograft without the need for structural bone grafting. A total of 110 consecutive dysplasia resurfacing arthroplasties in 103 patients (55 men and 48 women) performed between 1997 and 2000 was reviewed with a minimum follow-up of six years. The mean age at operation was 47.2 years (21 to 62) and 104 hips (94%) were Crowe grade II or III. During the mean follow-up of 7.8 years (6 to 9.6), three hips (2.7%) were converted to a total hip replacement at a mean of 3.9 years (2 months to 8.1 years), giving a cumulative survival of 95.2% at nine years (95% confidence interval 89 to 100). The revisions were due to a fracture of the femoral neck, a collapse of the femoral head and a deep infection. There was no aseptic loosening or osteolysis of the acetabular component associated with either of the revisions performed for failure of the femoral component. No patient is awaiting a revision. The median Oxford hip score in 98 patients with surviving hips at the final review was 13 and the 10th and the 90th percentiles were 12 and 23, respectively

Fifty-one infants with limited abduction of the hip and acetabular dysplasia were, between 1969 and 1975, treated with abduction-adduction exercises, administered by the parents; no abduction devices were used. In 1983 a follow-up examination was carried out on 41 of these patients. Although at birth these children had characteristics similar to patients with congenital dislocation of the hip, none of their hips dislocated. At birth acetabular measurements showed that half the children had severe dysplasia and the other half slight dysplasia; the difference between the affected and the healthy hips was significant. At follow-up the gait was normal in all the patients. Movements at the hips were symmetrical and within normal limits in all but one patient. The acetabular angle, the centre-edge angle, the shaft-neck angle, the hip ratio, and the size of the femoral head were the same on the previously affected side as on the normal side. Nineteen of the children followed up had preferred lying on one side and dysplasia of the upper hip had developed. The significance of the sleeping position on the development of acetabular dysplasia is discussed

A brief review of the literature on malignant change occurring in fibrous dysplasia is given and a further case of a sarcoma arising in a patient with polyostotic fibrous dysplasia is reported

Tibial dysplasia is a rare congenital deformity which must be distinguished from the more common fibular dysplasia. We have reviewed 24 patients with 35 affected legs. The classification system of Kalamchi and Dawe (1985) was found to be preferable to that of Jones, Barnes and Lloyd-Roberts (1978) as a guide to prognosis and management. We discuss the outcome of surgical treatment, recommending selective amputation for most cases

Aims. The influence of identifiable pre-operative factors on the outcome of eccentric rotational acetabular osteotomy (ERAO) is unknown. We aimed to determine the factors that might influence the outcome, in order to develop a scoring system for predicting the prognosis for patients undergoing this procedure. Patients and Methods. We reviewed 700 consecutive ERAOs in 54 men and 646 women with symptomatic acetabular dysplasia or early onset osteoarthritis (OA) of the hip, which were undertaken between September 1989 and March 2013. The patients’ pre-operative background, clinical and radiological findings were examined retrospectively. Multivariate Cox regression analysis was performed using the time from the day of surgery to a conversion to total hip arthroplasty (THA) as an endpoint. A risk score was calculated to predict the prognosis for conversion to THA, and its predictive capacity was investigated. Results. The congruity of the hip, age, the pre-operative minimum width of the joint space and range of abduction were identified as factors predicting conversion to THA. For three groups of patients (scoring 0 to 5, 6 to 7, and 8 to 9 points), the Kaplan-Meier event-free rates of survival at 15 years post-operatively for conversion to THA were 99.6%, 85.2% and 67.3%, respectively. Conclusion. These four pre-operative factors are easily measured and predict the prognosis for patients following ERAO. They may be used for decision making when offering surgical treatment to patients with acetabular dysplasia and early onset osteoarthritis. Cite this article: Bone Joint J 2016;98-B:1326–32

We examined clinically and radiologically the knees of 46 patients (27 females and 19 males) with diastrophic dysplasia. The age of the patients varied from newborn to 38 years. A total of 18 patients was followed during their growth until adolescence. The knees of two legally aborted fetuses appeared on examination to be macroscopically normal and congruous. Excessive valgus deformity of the tibiofemoral weight-bearing angle with a mean of 14° was noted in infancy. Most of the patients had marked instability of the knees. The range of movement of the knee began to decrease before the age of five years. There were signs of early degeneration and deformation of the bony epiphyses before the age of six years. The patellofemoral joint was abnormal from an early age. A marked patella infera, often associated with a lateral position of the patella with bony fragmentation, was noted. The knee in diastrophic dysplasia is basically unstable, showing early deformation of the subchondral bone and degeneration of the joint

Three amputated legs with tibial dysplasia were studied by radiography, arteriography and anatomical dissection. The radiographic appearances were the same as the Type 1b tibial dysplasia described by Jones, Barnes and Lloyd-Roberts (1978) in that the tibiae were absent but the lower femoral epiphyses were normal. However, our anatomical findings differed from those of Jones et al. since no bony or cartilaginous anlage of the proximal tibia was found in any of the three legs. The pattern of vascular anomaly was identical in the three legs and similar to the findings of Hootnick et al. (1980) in congenital short fibula. Congenital fusion of the subtalar joint was a constant finding. These results support the hypothesis that the arterial and skeletal systems are vulnerable to a teratogenic insult in the fifth week of embryonic life. The bony and arterial anomalies should be borne in mind by the surgeon attempting reconstructive surgery for this condition

We performed rotational acetabular osteotomy in order to treat dysplasia of the hip in five ambulatory adults with cerebral palsy. There was one man and four women, with a mean age of 21 years (16 to 27) who were followed up for a mean of 12 years and two months. The mean Sharp angle improved from 52° to 43°, the mean acetabular index from 30.2° to 2.8°, the mean centre-edge angle from −5.6° to 29.2°, and the mean acetabular head index from 49.2 to 88.2. There was no progression of joint degeneration and relief from pain was maintained. Our results suggest that rotational acetabular osteotomy is a valuable option for the treatment of acetabular dysplasia in adults with cerebral palsy who have incapacitating pain in the hip

We describe a new technique of reconstruction of the deficient acetabulum in cementless total hip arthroplasty. The outer iliac table just above the deficient acetabulum is osteotomised and slid downwards. We have termed this an iliac sliding graft. Between October 1997 and November 2001, cementless total hip arthroplasty with an iliac sliding graft was performed on 19 patients (19 hips) with acetabular dysplasia. The mean follow-up was 3.4 years (2 to 6). The mean pre-operative Harris hip score was 45.1 which improved significantly to 85.3 at the time of the final follow-up. No patient had post-operative abductor dysfunction. Incorporation of the graft was seen after two to three months in all patients. Resorption of the graft and radiolucencies were infrequent. This technique is a useful alternative to femoral head autografting when the patient’s own femoral head cannot be used

Hereditary multiple exostoses is an autosomal dominant disorder characterised by multiple osteochondromata, most commonly affecting the forearm, knee and ankle. Osteochondromata of the proximal femur have been reported to occur in 30% to 90% of affected patients with coxa valga in 25%. Acetabular dysplasia is rare but has been described. This is the first report of a patient requiring surgical intervention. A girl was seen at the age of nine with hereditary multiple exostoses and when 12 developed bilateral pain in the groin. Radiographs showed severely dysplastic acetabula with less than 50% coverage of the femoral heads and widening of the medial joint space. Large sessile osteochondromata were present along the medial side of the femoral neck proximal to the lesser trochanter, with associated coxa valga. The case illustrates the importance of obtaining initial skeletal surveys in children with hereditary multiple exostoses to identify potential problems such as acetabular dysplasia and subluxation of the hip

We investigated the variables which determine the outcome after triple osteotomy of the pelvis for the treatment of congenital dysplasia of the hip. We reviewed 51 patients (61 hips) with a median age at operation of 23 years who were treated with a Tönnis triple osteotomy. The median follow-up was six years with a minimum of two years. Eight patients (eight hips) required a revision procedure. Of the remaining 53 hips, the results were good or excellent in 36 (68%) when evaluated according to the Harris hip score (median 90 points), and 33 patients (65%) were satisfied with the procedure. Logistic regression analysis indicated that the incidence of complications such as nonunion at an osteotomy site influenced patient satisfaction (p = 0.079). The incidence of complications correlated positively with increasing patient age at operation (p = 0.004). The amount of acetabular correction did not correlate with patient satisfaction. In univariate analysis, the groups of ’satisfied’ and ‘not satisfied’ patients differed significantly in Harris hip score, age, incidence of nonunion at the osteotomy sites, complications and late revisions. In conclusion, the patient’s age at operation and the incidence of complications influence patient satisfaction after triple osteotomy, but the amount of radiologically evident acetabular correction shows no correlation to outcome

1. Two cases are reported showing the syndrome of chondrodysplasia, manual polydactyly, ectodermal dysplasia affecting the teeth and nails, and congenital heart disease. 2. Particulars of thirty-eight cases are tabulated, and the features of the syndrome are discussed. 3. The syndrome is regarded as showing an autosomal recessive mode of inheritance

We report the case of a 12-year-old boy with polyostotic fibrous dysplasia of the upper femur in whom a massive customised polyethylene prosthesis functioned successfully for more than fifty years

The exceptionally high prevalence of diastrophic dysplasia in Finland has enabled us to analyse the foot deformities of 102 patients at their first orthopaedic evaluation and classify 204 feet into five categories. The most common finding (43%) was a foot with tarsal valgus deformity and metatarsus adductus; 37% showed either equinovarus adductus (29%) or equinus (8%) deformities. At the first examination 13% showed metatarsus adductus deformity alone, and 7% were clinically normal. The expression 'club foot', generally used for the foot deformity in diastrophic dysplasia is a misnomer. There is a wide spectrum of deformities, some of them specific for the condition

1. A survey of genetic and other etiological factors has been carried out in 589 index patients with congenital dislocation of the hip and their families, with special investigation of acetabular dysplasia, familial joint laxity and a comparison of neonatal and late-diagnosis cases. 2. It is believed that there are two etiological groups with congenital dislocation of the hip, i) a group with acetabular dysplasia which is inherited as a multiple gene system and is responsible for a high proportion of cases diagnosed late, and ii) a group with joint laxity which is responsible for a high proportion of neonatal cases. 3. Evidence is presented to show that acetabular dysplasia is a separate heritable system in some families. 4. Other findings relating to the genetic aspects of the survey are summarised

The acetabular rim syndrome is a pathological entity which we illustrate by reference to 29 cases. The syndrome is a precursor of osteoarthritis of the hip secondary to acetabular dysplasia. The symptoms are pain and impaired function. All our cases were treated by operation which consisted in most instances of re-orientation of the acetabulum by peri-acetabular osteotomy and arthrotomy of the hip. In all cases, the limbus was found to be detached from the bony rim of the acetabulum. In several instances there was a separated bone fragment, or 'os acetabuli' as well. In acetabular dysplasia, the acetabular rim is subject to abnormal stress which may cause the limbus to rupture, and a fragment of bone to separate from the adjacent bone margin. Dysplastic acetabuli may be classified into two radiological types. In type I there is an incongruent shallow acetabulum. In type II the acetabulum is congruent but the coverage of the femoral head is deficient

Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia in the ilium. We believe this to be the second reported case at this site. Treatment was by excision of the hemipelvis including the ala of the sacrum. A review of the literature is presented